Abstract 3542: Presentation and Outcome of Marfan’s Syndrome Patients with Dissection and Thoracoabdominal Aortic Aneurysm
Introduction: Much attention is directed towards the aortic root in Marfan’s syndrome, but there is a paucity of data regarding intervention criteria for the thoraco-abdominal aorta (TAA). We report a consecutive series of Marfan’s syndrome patients with type B dissection.
Method: A retrospective analysis of 22 Marfan’s syndrome patients with type B dissection managed between September 1999 and April 2006 was performed. The serial diameters and linear expansion rates were calculated from the CT images and outcome of intervention was analysed.
Results: There were 14 male patients. The median age was 38.5 years (range 23– 61). Nineteen had prior aortic surgery or endovascular stenting. Two patients required surgery at initial acute dissection presentation, 1 underwent surgery (Arch + TAA replacement) for failed stenting and 1 required gastric resection for coeliac axis malperfusion. During follow-up, surgery was recommended in 20 patients and undertaken in 19 (1 ruptured and died prior to operation). Of the 19 operated patients, 2 presented with rupture, 2 with airway obstruction, 1 with intermittent paraplegia and 14 underwent planned surgery for increased expansion rate ± pain. All patients had residual type A or chronic type B dissection. Median aortic dimension at surgery was 6.7 cm (IQR 6.6- 9.0). The pre-operative mean expansion rate increased from the 0.7 cm/year to 1.7 cm/year (p=0.005), in the year prior to operation. Fourteen patients underwent Crawford extent II repair, 3 extent I and 2 extent III repair. Profound hypothermia and CSF drainage was used in 16 and 18 patients respectively. There was no early mortality, paraplegia or renal failure. The mean cardiopulmonary bypass and hypothermic circulatory arrest time 231 and 21 minutes respectively. After a median follow-up of 39 months (range1–79), there remains 100% survival.
Conclusion: TAAA repair in Marfan’s syndrome can be performed safely with good outcomes. Intervention criteria should be reconsidered to prevent the risk of rupture or emergency presentation. Any role of endovascular management needs careful consideration.